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Clinical Outcomes Among Immunotherapy-Treated Patients With Primary Cardiac Soft Tissue Sarcomas: A Multicenter
Amin H Nassar1, Edward El-Am2, Ryan Denu3
1Yale University School of Medicine, New Haven, Connecticut, USA.
Background:
Primary cardiac soft tissue sarcomas (CSTS) affect young adults, with dismal outcomes.
Objectives:
The aim of this study was to investigate the clinical outcomes of patients with CSTS receiving immune checkpoint inhibitors (ICIs).
Methods:
A retrospective, multi-institutional cohort study was conducted among patients with CSTS between 2015 and 2022. The patients were treated with ICI-based regimens. The Kaplan-Meier method was used to estimate overall survival (OS) and progression-free survival (PFS). Objective response rates were determined according to Response Evaluation Criteria in Solid Tumors version 1.1. Treatment-related adverse events were graded per the Common Terminology Criteria for Adverse Events version 5.0.
Results:
Among 24 patients with CSTS, 17 (70.8%) were White, and 13 (54.2%) were male. Eight patients (33.3%) had angiosarcoma. At the time of ICI treatment, 18 patients (75.0%) had metastatic CSTS, and 4 (16.7%) had locally advanced disease. ICIs were administered as the first-line therapy in 6 patients (25.0%) and as the second-line therapy or beyond in 18 patients (75.0%). For the 18 patients with available response data, objective response rate was 11.1% (n = 2 of 18). The median PFS and median OS in advanced and metastatic CSTS (n = 22) were 5.7 months (95% CI: 2.8-13.3 months) and 14.9 months (95% CI: 5.7-23.7 months), respectively. The median PFS and OS were significantly shorter in patients with cardiac angiosarcomas than in those with nonangiosarcoma CSTS: median PFS was 1.7 vs 11 months, respectively (P < 0.0001), and median OS was 3.0 vs 24.0 months, respectively (P = 0.008). Any grade treatment-related adverse events occurred exclusively in the 15 patients with nonangiosarcoma CSTS (n = 7 [46.7%]), of which 6 (40.0%) were grade ≥3.
Conclusions:
Although ICIs demonstrate modest activity in CSTS, durable benefit was observed in a subset of patients with nonangiosarcoma, albeit with higher toxicity.
Insights
Immune checkpoint inhibitors (ICIs) show limited efficacy in primary cardiac soft tissue sarcomas (CSTS), with a low response rate. However, a subset of patients with non-angiosarcoma CSTS experienced durable benefits, despite increased toxicity.
Area of Science:
- Oncology
- Immunotherapy
- Cardiovascular Research
Background:
- Primary cardiac soft tissue sarcomas (CSTS) are rare and aggressive tumors predominantly affecting young adults.
- Outcomes for patients with CSTS are historically poor, highlighting the need for novel therapeutic strategies.
Purpose of the Study:
- To evaluate the clinical efficacy and safety of immune checkpoint inhibitors (ICIs) in patients diagnosed with CSTS.
- To determine the impact of ICIs on overall survival (OS) and progression-free survival (PFS) in this patient population.
Main Methods:
- A retrospective, multi-institutional cohort study analyzed patients with CSTS treated with ICI-based regimens between 2015 and 2022.
- Kaplan-Meier analysis was employed to estimate OS and PFS. Response evaluation followed RECIST v1.1 criteria, and adverse events were graded using CTCAE v5.0.
Main Results:
- The objective response rate to ICIs was 11.1% among 18 evaluable patients with advanced/metastatic CSTS.
- Median PFS and OS for advanced/metastatic CSTS were 5.7 and 14.9 months, respectively.
- Patients with non-angiosarcoma CSTS showed significantly longer PFS (11 vs 1.7 months) and OS (24 vs 3.0 months) compared to those with angiosarcoma. Toxicity was noted primarily in the non-angiosarcoma group.
Conclusions:
- Immune checkpoint inhibitors (ICIs) exhibit modest activity in primary cardiac soft tissue sarcomas (CSTS).
- A subset of patients with non-angiosarcoma CSTS may achieve durable responses to ICIs, though this is associated with increased toxicity.
- Further research is warranted to optimize ICI therapy for CSTS, particularly for angiosarcoma subtypes.
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